Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

Pumin Zhang, Nanette J. Liégeois, Calvin Wong, Milton Finegold, Harry Hou, Janet C. Thompson, Adam Silverman, J. Wade Harper, Ronald A. DePinho, Stephen J. Elledge

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613 Scopus citations

Abstract

Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal modullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.

Original languageEnglish (US)
Pages (from-to)151-158
Number of pages8
JournalNature
Volume387
Issue number6629
DOIs
StatePublished - May 8 1997

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    Zhang, P., Liégeois, N. J., Wong, C., Finegold, M., Hou, H., Thompson, J. C., Silverman, A., Harper, J. W., DePinho, R. A., & Elledge, S. J. (1997). Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome. Nature, 387(6629), 151-158. https://doi.org/10.1038/387151a0